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James L. Leach, Lili Miles, David M. Henkel, Hansel M. Greiner, Marcia K. Kukreja, Katherine D. Holland, Douglas F. Rose, Bin Zhang and Francesco T. Mangano

Object

The authors conducted a study to correlate histopathological features, MRI findings, and postsurgical outcomes in children with cortical dysplasia (CD) by performing a novel resection site–specific evaluation.

Methods

The study cohort comprised 43 children with intractable epilepsy and CD. The MR image review was blinded to pathology but with knowledge of the resection location. An MRI score (range 0–7) was calculated for each resection region based on the number of imaging features of CD and was classified as “lesional” or “nonlesional” according to all imaging features. Outcome was determined using the International League Against Epilepsy (ILAE) scale. The determination of pathological CD type was based on the ILAE 2011 consensus classification system, and the cortical gliosis pattern was assessed on GFAP staining.

Results

There were 89 resection regions (50 ILAE Type I, 29 Type IIa, and 10 Type IIb). Eleven (25.6%) of 43 children had more than one type of CD. The authors observed MRI abnormalities in 63% of patients, characteristic enough to direct resection (lesional) in 42%. Most MRI features, MRI score ≥ 3, and lesional abnormalities were more common in patients with Type II CD. Increased cortical signal was more common in those with Type IIb (70%) rather than Type IIa (17.2%) CD (p = 0.004). A good outcome was demonstrated in 39% of children with Type I CD and 72% of those with Type II CD (61% in Type IIa and 100% in Type IIb) (p = 0.03). A lesional MRI abnormality and an MRI score greater than 3 correlated with good outcome in 78% and 90% of patients, respectively (p < 0.03). Diffuse cortical gliosis was more prevalent in Type II CD and in resection regions exhibiting MRI abnormalities. Complete surgical exclusion of the MRI abnormality was associated with a better postoperative outcome.

Conclusions

This study provides a detailed correlation of MRI findings, neuropathological features, and outcomes in children with intractable epilepsy by using a novel resection site–specific evaluation. Because 25% of the patients had multiple CD subtypes, a regional analysis approach was mandated. Those children with lesional MRI abnormalities, Type II CD, and surgical exclusion of the MRI abnormality had better outcomes. Type II CD is more detectable by MRI than other types, partly because of the greater extent of associated gliosis in Type II. Although MRI findings were correlated with the pathological CD type and outcome in this study, the majority of patients (58%) did not have MRI findings that could direct surgical therapy, underscoring the need for improved MRI techniques for detection and for the continued use of multimodal evaluation methods in patient selection.

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Karen L. Skjei, Ephraim W. Church, Brian N. Harding, Mariarita Santi, Katherine D. Holland-Bouley, Robert R. Clancy, Brenda E. Porter, Gregory G. Heuer and Eric D. Marsh

OBJECT

Mutations in the sodium channel alpha 1 subunit gene (SCN1A) have been associated with a wide range of epilepsy phenotypes including Dravet syndrome. There currently exist few histopathological and surgical outcome reports in patients with this disease. In this case series, the authors describe the clinical features, surgical pathology, and outcomes in 6 patients with SCN1A mutations and refractory epilepsy who underwent focal cortical resection prior to uncovering the genetic basis of their epilepsy.

METHODS

Medical records of SCN1A mutation-positive children with treatment-resistant epilepsy who had undergone resective epilepsy surgery were reviewed retrospectively. Surgical pathology specimens were reviewed.

RESULTS

All 6 patients identified carried diagnoses of intractable epilepsy with mixed seizure types. Age at surgery ranged from 18 months to 20 years. Seizures were refractory to surgery in every case. Surgical histopathology showed evidence of subtle cortical dysplasia in 4 of 6 patients, with more neurons in the molecular layer of the cortex and white matter.

CONCLUSIONS

Cortical resection is unlikely to be beneficial in these children due to the genetic defect and the unexpected neuropathological finding of mild diffuse malformations of cortical development. Together, these findings suggest a diffuse pathophysiological mechanism of the patients’ epilepsy which will not respond to focal resective surgery.

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Ravindra Arya, Jeffrey R. Tenney, Paul S. Horn, Hansel M. Greiner, Katherine D. Holland, James L. Leach, Michael J. Gelfand, Leonid Rozhkov, Hisako Fujiwara, Douglas F. Rose, David N. Franz and Francesco T. Mangano

OBJECT

Tuberous sclerosis complex (TSC) with medically refractory epilepsy is characterized by multifocal brain abnormalities, traditionally indicating poor surgical candidacy. This single-center, retrospective study appraised seizurerelated, neuropsychological, and other outcomes of resective surgery in TSC patients with medically refractory epilepsy, and analyzed predictors for these outcomes.

METHODS

Patients with multilesional TSC who underwent epilepsy surgery between 2007 and 2012 were identified from an electronic database. All patients underwent multimodality noninvasive and subsequent invasive evaluation. Seizure outcomes were classified using the International League Against Epilepsy (ILAE) scale. The primary outcome measure was complete seizure remission (ILAE Class 1). Secondary outcome measures included 50% responder rate, change in full-scale IQ, electroencephalography improvement, and reduction in antiepileptic drug (AED) burden.

RESULTS

A total of 37 patients with TSC underwent resective surgery during the study period. After a mean follow-up of 5.68 ± 3.67 years, 56.8% achieved complete seizure freedom (ILAE Class 1) and 86.5% had ILAE Class 4 outcomes or better. The full-scale IQ on follow-up was significantly higher in patients with ILAE Class 1 outcome (66.70 ± 12.36) compared with those with ILAE Class 2 or worse outcomes (56.00 ± 1.41, p = 0.025). In 62.5% of the patients with ILAE Class 2 or worse outcomes, the number of AEDs were found to be significantly reduced (p = 0.004).

CONCLUSIONS

This study substantiates the evidence for efficacy of resective epilepsy surgery in patients with bilateral multilesional TSC. More than half of the patients were completely seizure free. Additionally, a high proportion achieved clinically meaningful reduction in seizure burden and the number of AEDs.

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Ravindra Arya, Francesco T. Mangano, Paul S. Horn, Sabrina K. Kaul, Serena K. Kaul, Celie Roth, James L. Leach, Michele Turner, Katherine D. Holland and Hansel M. Greiner

OBJECTIVE

There is emerging data that adults with temporal lobe epilepsy (TLE) without a discrete lesion on brain MRI have surgical outcomes comparable to those with hippocampal sclerosis (HS). However, pediatric TLE is different from its adult counterpart. In this study, the authors investigated if the presence of a potentially epileptogenic lesion on presurgical brain MRI influences the long-term seizure outcomes after pediatric temporal lobectomy.

METHODS

Children who underwent temporal lobectomy between 2007 and 2015 and had at least 1 year of seizure outcomes data were identified. These were classified into lesional and MRI-negative groups based on whether an epilepsy-protocol brain MRI showed a lesion sufficiently specific to guide surgical decisions. These patients were also categorized into pure TLE and temporal plus epilepsies based on the neurophysiological localization of the seizure-onset zone. Seizure outcomes at each follow-up visit were incorporated into a repeated-measures generalized linear mixed model (GLMM) with MRI status as a grouping variable. Clinical variables were incorporated into GLMM as covariates.

RESULTS

One hundred nine patients (44 females) were included, aged 5 to 21 years, and were classified as lesional (73%), MRI negative (27%), pure TLE (56%), and temporal plus (44%). After a mean follow-up of 3.2 years (range 1.2–8.8 years), 66% of the patients were seizure free for ≥ 1 year at last follow-up. GLMM analysis revealed that lesional patients were more likely to be seizure free over the long term compared to MRI-negative patients for the overall cohort (OR 2.58, p < 0.0001) and for temporal plus epilepsies (OR 1.85, p = 0.0052). The effect of MRI lesion was not significant for pure TLE (OR 2.64, p = 0.0635). Concordance of ictal electroencephalography (OR 3.46, p < 0.0001), magnetoencephalography (OR 4.26, p < 0.0001), and later age of seizure onset (OR 1.05, p = 0.0091) were associated with a higher likelihood of seizure freedom. The most common histological findings included cortical dysplasia types 1B and 2A, HS (40% with dual pathology), and tuberous sclerosis.

CONCLUSIONS

A lesion on presurgical brain MRI is an important determinant of long-term seizure freedom after pediatric temporal lobectomy. Pediatric TLE is heterogeneous regarding etiologies and organization of seizure-onset zones with many patients qualifying for temporal plus nosology. The presence of an MRI lesion determined seizure outcomes in patients with temporal plus epilepsies. However, pure TLE had comparable surgical seizure outcomes for lesional and MRI-negative groups.